..A conceptual framework and pipeline for evaluating the large number of potential HD biomarkers is presented, and the need for systematic head-to-head comparison of candidate markers is highlighted...

..Our aim was to identify sensitive and reliable biomarkers in premanifest carriers of mutated HTT and in individuals with early HD that could provide essential methodology for the assessment of therapeutic interventions...

Relationship between CAG repeat length and brain volume in premanifest and early Huntington's disease

..Overall we have demonstrated that increased CAG repeat length is associated with atrophy in extra-striatal as well as striatal regions, which has implications for the monitoring of disease-modifying therapies in the condition...

PINK1-associated Parkinson's disease is caused by neuronal vulnerability to calcium-induced cell death

..Our study reveals specific associations between atrophy and decline in a range of clinical modalities, demonstrating the utility of VBM correlation analysis for investigating these relationships in HD...

The progression of regional atrophy in premanifest and early Huntington's disease: a longitudinal voxel-based morphometry study

..We conclude that the study of whole-brain atrophy has the potential to inform our understanding of the neurobiology of HD and warrants further study as one means of assessing the outcomes of future clinical trials...

C9orf72 expansions are the most common genetic cause of Huntington disease phenocopies

..These data suggest that pathologically activated microglia in AST and other areas related to cognitive function, maybe better predictors of clinical onset and stresses the importance of early cognitive assessment in HD...

Abnormal motor cortex plasticity in premanifest and very early manifest Huntington disease

..Even in early HD there is a wide-ranging impairment in recognition of negative emotions denoting 'threat'. Our findings implicate a generic fronto-subcortical network in the pathogenesis of these emotion recognition deficits...

Automated quantification of caudate atrophy by local registration of serial MRI: evaluation and application in Huntington's disease

..We describe and evaluate an automated technique based on a local registration and boundary shift integral (BSI) approach at the caudate-CSF and caudate-white matter boundaries; caudate boundary shift integral (CBSI)...

The potential of composite cognitive scores for tracking progression in Huntington's disease

..Composite scores derived from joint statistical modelling of individual risk factors are widely used to identify individuals who are at increased risk of developing disease or of faster disease progression...

Quality of life in Huntington's disease: a comparative study investigating the impact for those with pre-manifest and early manifest disease, and their partners

..The current results demonstrate that the deterioration of executive functioning in HD is variable and that some types of executive processing might already be impaired in early HD, whereas others remain intact...

..However, given evidence of posterior cortical atrophy in premanifest HD, we predicted visuomotor integration would be adversely affected, with greater impairment under conditions of indirect visual feedback...

Abnormal motor cortex excitability in preclinical and very early Huntington's disease

..We briefly touch on the technologies used to make these models, and then focus on recent results from new models. We discuss why such models are useful when they do - and do not - mimic the human disorder...

Hypothalamic involvement in Huntington's disease: an in vivo PET study

..The results suggest that hemolysis may be linked to the pathogenesis of Huntington's disease and that assay of hemoglobin and alpha1-microglobulin may provide biomarkers that are linked to biologically relevant processes...

Stability effects on results of diffusion tensor imaging analysis by reduction of the number of gradient directions due to motion artifacts: an application to presymptomatic Huntington's disease

..This finding has important implications for the aetiology of sporadic AD, and for other apparently sporadic neurodegenerative diseases such as Parkinson's disease, motor neuron disease and Creutzfeldt-Jakob disease...

Mark KristiansenMedical Research Council Prion Unit and Department of Neurodegenerative Disease, Institute of Neurology, University College London, Queen Square, London WC1N 3BG, United KingdomJ Biol Chem 280:38851-61. 2005

..This, in turn, triggers caspase-dependent apoptosis and further implicates proteasome dysfunction in the pathogenesis of prion diseases...

Prevalence of adult Huntington's disease in the UK based on diagnoses recorded in general practice records

Stephen J W EvansDepartment of Epidemiology, London School of Hygiene and Tropical Medicine, University of London, London, UKJ Neurol Neurosurg Psychiatry 84:1156-60. 2013

..Recently, it has been suggested that the prevalence may be substantially greater than previously reported. This study was undertaken to estimate the overall UK prevalence in adults diagnosed with HD, using data from primary care...

The ubiquitin-proteasome system in neurodegeneration

Chris McKinnonDepartment of Neurodegenerative Disease, University College London Institute of Neurology, London, United KingdomAntioxid Redox Signal 21:2302-21. 2014

..To date, no therapies have been developed that can specifically up-regulate this system...

Targets for future clinical trials in Huntington's disease: what's in the pipeline?

..Building on a tradition of collaborative research in HD, great advances have been made in the field since that time and a range of outcome measures are now being recommended in order to assess efficacy in future therapeutic trials...

..Proteins of interest were evaluated using immunoblotting and ELISA in plasma from 2 populations, CSF and R6/2 mice. The identified proteins demonstrate neuroinflammation in HD and warrant further investigation as possible biomarkers...